Nephrotic syndrome

Nephrotic syndrome is defined as a complex of nephrological symptoms dominated by severe proteinuria.

Symptoms

Proteinuria
	All subtypes of congenital nephrotic syndrome are characterized by mostly nephrotic range (more than 3,5g/d) proteinuria.

Systematic

Hereditary glomerular disease
	Fibronectin glomerulopathy
	Glomerulonephritis
	Glomerulosclerosis
	Lipoprotein glomerulopathy
	Myoclonus-nephropathy syndrome
	Nephritic syndrome
	Nephrotic syndrome
		Congenital nephrotic syndrome type 01 (Finnish type)
			NPHS1
		Congenital nephrotic syndrome type 02
			NPHS2
		Congenital nephrotic syndrome type 03
			PLCE1
		Congenital nephrotic syndrome type 04
			WT1
		Congenital nephrotic syndrome type 05 (Pierson syndrome)
			LAMB2
		Congenital nephrotic syndrome type 06
			PTPRO
		Congenital nephrotic syndrome type 07
			DGKE
		Congenital nephrotic syndrome type 08
			ARHGDIA
		Congenital nephrotic syndrome type 09
			COQ8B
		Congenital nephrotic syndrome type 10
			EMP2
		Congenital nephrotic syndrome type 11
			NUP107
		Congenital nephrotic syndrome type 12
			NUP93
		Congenital nephrotic syndrome type 13
			NUP205
		Congenital nephrotic syndrome type 14
			SGPL1
		Congenital nephrotic syndrome type 15
			MAGI2
		Congenital nephrotic syndrome type 16
			KANK2
		Congenital nephrotic syndrome type 17
			NUP85
		Congenital nephrotic syndrome type 18
			NUP133
		Congenital nephrotic syndrome type 19
			NUP160
		Congenital nephrotic syndrome type 20
			TBC1D8B
		Congenital nephrotic syndrome type 21
			AVIL
		Congenital nephrotic syndrome type 22
			NOS1AP
		Congenital nephrotic syndrome type 23
			KIRREL1
		Congenital nephrotic syndrome type 24
			DAAM2
		Donnai-Barrow syndrome
			LRP2
		GPC5
		Glomerulotubular nephropathy
			FAT1
		Interstitial lung disease with nephrotic syndrome and epidermolysis bullosa
			ITGA3
		Lipoprotein glomerulopathy
			APOE
		Schimke Immunoosseous dysplasia
			SMARCAL1
		XPO5

References:

1.	Weber S et al. (2005) Recurrence of focal-segmental glomerulosclerosis in children after renal transplantation: clinical and genetic aspects.

2.	None (2003) Podocyte differentiation and hereditary proteinuria/nephrotic syndromes.

3.	None (2003) Approach to the evaluation of heritable diseases and update on familial focal segmental glomerulosclerosis.

4.	Tryggvason K et al. (2006) Hereditary proteinuria syndromes and mechanisms of proteinuria.

5.	Orphanet article Orphanet ID 1830

6.	Wikipedia article Wikipedia EN (Nephrotic_syndrome)

Update: June 23, 2025

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